Infantile Spasms, Clinical Manifestations of a Rare Brain Tumor: A Case Report and Literature Review

Objectives Desmoplastic infantile ganglioglioma (DIG) has a favorable prognosis and is classified as a benign infantile brain tumor. The DIG is more common in children under 2 years of age than in other age groups. This report introduces a 5.5 month-old infant who was referred with infantile spasms and diagnosed with a brain tumor. Brain magnetic resonance imaging showed a large heterogeneous mass in the right hemisphere with shifting to the other side. The patient underwent surgery. The extra-axial mass was completely resected, and the diagnosis of DIG grade I was confirmed by pathology. After one year, patient development was normal, and the seizures did not recur. In addition, the general condition was good. With a brief review and search in the literature, 13 case reports were identified 9 of which were male cases. The mean age of initial manifestation to final tumor diagnosis was 4 months. Out of 13 patients, 8 cases were reported with the mass origin in the right hemisphere. The most commonly observed tumors were glioma (n=4) and hypothalamic hamartoma (n=3). Except for three patients who died, the remaining had a complete recovery after tumor removal with a seizure-free interval at follow-up.

Desmoplastic infantile ganglioglioma (DIG) is a rare infantile brain tumor that rarely appears with infantile spasms. The DIG (also called desmoplastic infantile astrocytoma of infancy) is primarily observed in infants and young children, with a male predominance. Patients with DIGs typically present in infancy with macrocephaly or partial complex seizures. The median reported age of presentation is about 5 months. The involvement of multiple lobes is common with a predilection for the frontal and parietal lobes (2).
Vandenberg was the first who described DIG in 1987 (3). The DIG is an uncommon intracranial tumor that, despite the massive size and severe desmoplasia with astrocytic and ganglionic cells, is benign and can occur in an infant at the age of fewer than 18 months. The result of the DIG tumor's surgical resection has been successful to date (2,4).

Most of the present studies have reported that
infantile spasms have appeared with central nervous system diseases; however, the occurrence of infantile spasms with brain tumors is not common (4). In this report, we present a rare case of infantile brain tumor that clinically presents with infantile spasms and have an overview on similar cases reported in the literature.

Case Report
The patient, a 5.5-month-old infantile male, was born after an uneventful full-term pregnancy and a natural vaginal delivery. His birth weight and head circumference were 3200 g and 34 cm,  According to gender, the patients included nine male and four female cases (6)(7)(8)(9)(10)(11)(12)(13). The mean age of tumor diagnosis from the onset of symptoms to diagnosis was within 2 weeks to 2 months, which was 2 weeks in the present case. Except for three patients, the duration of diagnosis for each of them was 9 (11), 18 (12), and 21 (13) months.  The origin of the mass in three patients was the left hemisphere (7,9,10). In one patient, the mass was in the hypothalamus (6). In another patient, the mass was in the posterior fossa (13).

Of 13 patients, 8 patients were reported with
In the present case, the source of the mass was the right hemisphere, compatible with the most common sites. Pathologically, the most commonly observed tumors were glioma (n=4) (8,10,11) and hypothalamus hamartoma (n=3) (6,7,9). Except for three patients who died (the first case from glioma grade III [11], the second case from medulloepithelioma [13], and the third case from ganglioglioma [8]) and one patient with primitive neuroectodermal tumor (12) who suffered from psychomotor regression, the remaining patients had a complete recovery with complete control of seizures after the surgical resection of the mass.
The present case also had complete seizure control within 9 months of follow-up after tumor removal, and her development was normal.
This study received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.

Author's Contribution
All authors had full access to the data, contributed to the study, approved the final version of the manuscript, and took responsibility for its accuracy and integrity.

Conflicts of Interest
The authors have no conflict of interest in preparing and submitting this report.